POS1307 HEMATOLOGICAL MANIFESTATIONS IN THE PRESENCE OF ANTIPHOSPHOLIPID ANTIBODIES IN A PEDIATRIC COHORT
Authors: P. Morán Álvarez, Á. Andreu-Suárez, L. R. Caballero Motta, J. Calzada, S. Gassiot, R. Berrueco, L. Giovannelli, A. Boteanu, M. Vázquez Díaz, J. Anton, C. Bracaglia, F. De Benedetti
Journal: Annals of the Rheumatic Diseases (2022)

Background Antiphospholipid syndrome (APS) is an autoimmune disease characterized by thrombotic events (TEs) and/or pregnancy morbidity, in association with two consecutive positive determinations (at least 12 weeks apart) of antiphospholipid antibodies (aPLs). Several manifestations which are not considered clinical criteria of APS have been identified, including hematologic disorders. 1 Objectives To identify the different variables associated with the development of hematological manifestations in the presence of aPLs in a pediatric cohort. Methods Multicentric historical cohort of children ≤ 18 years from three Spanish and one Italian tertiary referral hospitals with at least 2 positive determinations (≥12 weeks apart) of IgG/IgM anticardiolipin (aCL), IgG/IgM anti β2-glycoprotein I (aβ2GP) and/or LA. Demographic, clinical and analytical data were collected. A bivariate and multivariate analysis were carried out. Multicollinearity was also explored to build the model. Results 131 children were included. Demographic, clinical, analytical data; and results of bivariant analysis are shown in Table 1. Table 1. Demographic characteristics; non-thrombotic clinical manifestations; immunologic laboratory findings; and bivariate analysis according to each specific entity and the occurrence of “hematological” manifestations. Categorical variables expressed as number (n) and percentage (%); Numerical variables with normal distribution expressed as mean and SD (standard deviation). * p&lt;0.05. Variables Totaln= 131 Hematological manifestationsn= 55 Absence of hematological manifestationsn= 76 P-value Sex, female, n (%) 85 (64.9) 39 (70.9) 46 (60.5) 0.219 Age, years, mean (SD) 10.9 (5.1) 10.8 (5.03) 10.9 (5.2) 0.974 Rage, n (%) 0.126 Caucasian 105 (81.4) 43 (79.6) 62 (82.7) Maghreb 9 (7) 2 (3.7) 7 (9.3) Latinoamerican 8 (6.2) 6 (11.1) 2 (2.7) Asian 5 (3.9) 3 (5.6) 2 (2.7) Family history of AIDs, n (%) 33 (25.2) 17 (33.3) 16 (21.1) 0.122 SLE diagnosis, n (%) 34 (26) 20 (36.4) 14 (18.4) 0.021* Thrombotic events, n (%) 16 (12.2) 7 (12.7) 9 (11.8) 0.879 Hematological, n (%) 55 (42) - - - Thrombocytopenia 30 (22.9) Leucopenia 16 (8.4) Hemolytic anemia 7 (5.3) Evans syndrome 2 (1.2) Neurological, n (%) 26 (20) 10 (18.2) 16 (21.3) 0.657 Chorea 8 (6.1) Epilepsy 5 (3.8) Migraine 4 (3) Cutaneous, n (%) 23 (17.6) 6 (10.9) 17 (22.4) 0.089 Raynaud 17 (13) Livedo 5 (3.1) Cardiac, n (%) 22 (16.8) 12 (21.8) 10 (13.2) 0.191 Renal, n (%) 2 (1.5) 0 (0) 2 (2.6) 0.225 aCL, n (%) IgG 54 (41.2) 24 (43.6) 30 (39.5) 0.633 IgM 31 (23.7) 20 (36.4) 11 (14.5) 0.004* aB2GP, n (%) IgG 59 (45) 29 (52.7) 30 (39.5) 0.132 IgM 24 (18.3) 16 (29.1) 8 (10.5) 0.007* LA, n (%) 76 (58.5) 37 (67.3) 39 (52) 0.081 aPL profile, n (%) Simple 76 (58) 25 (45.5) 51 (67.1) 0.008* Double 30 (22.9) 13 (23.6) 17 (22.4) Triple 25 (19.1) 17 (30.9) 8 (10.5) ANA +, n (%) 72 (59.5) 33 (66) 39 (54.9) 0.222 The multivariate analysis identified as independent risk factors to develop a hematological manifestation, children with: SLE diagnosis [OR 1.2, 95 CI (0.5-5.8), p= 0.015], cutaneous manifestations [OR 1.01, 95 CI (0.6 -2.8), p= 0.091], LA + [OR 0.8, 95 CI (0.4 -3.6), p= 0.058], and IgG aβ2GP + [OR 0.9, 95 CI (0.4 - 3.9), p= 0.048]. Sex, age, familiar history of AIDs or IgM B2GP+ did not show a higher risk. Conclusion Non-criteria manifestations, specially hematologic disorders, are the most frequent events in the presence of aPLs in our cohort. Cutaneous manifestations, a positive personal history of SLE, LA positivity and IgG aβ2GP positivity were associated with a higher risk of developing hematological manifestations. Therefore, their inclusion as APS classification criteria should be considered. References [1]Abreu MM, Danowski A, Wahl DG, et al. The relevance of “non-criteria” clinical manifestations of antiphospholipid syndrome: 14th International Congress on Antiphospholipid Antibodies Technical Task Force Report on Antiphospholipid Syndrome Clinical Features. Autoimmun Rev. 2015 Disclosure of Interests None declared